Congenital rubella syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: :Kalsang Dolma, M.B.B.S.[2]; Aditya Govindavarjhulla, M.B.B.S. [3]

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Overview

Historical Perspective

Originally, rubella was referred to as the third disease, because it was thought to branch from measles or Scarlet fever. However, in 1814, it was recognized as a separate entity of disease and became known as German measles.[1][2] In 1941, congenital rubella syndrome was recognized as a complication of rubella infection among pregnant women who became infected with the virus early in their pregnancy.[1][2] An Australian ophthalmologist made the connection and reported 78 cases of congenital cataract among infants born to mothers who had a rubella infection early in their pregnancy.[1][2]

Pathophysiology

Classification

There is no classification scheme for congenital rubella syndrome. However, in 2009, the CDC published the case classification of congenital rubella syndrome and divided into suspected cases, probable cases, confirmed cases and infection only, based on symptoms and/or laboratory findings.[3]

Causes

The cause of congenital rubella syndrome is rubella virus. Rubella virus is a single stranded, positive sense RNA virus (ssRNA). It is the only member of the genus Rubivirus and belongs to the family of Togaviridae.

Differentiating Congenital Rubella Syndrome from other Diseases

The most important congenital infections, which can be transmitted vertically from mother to fetus are the TORCH infections. These infections have overlapping features and hence, must be differentiated from CRS.[4][5]

Epidemiology and Demographics

The exact incidence of congenital rubella syndrome is hard to estimate, because in many parts of the developing world rubella has been eliminated. However, very few cases are still reported in the U.S and they are believed to be imported cases. In addition, rubella infection and congenital rubella syndrome are still a challenge in the developing world, with most cases occurring in Africa and Southeast Asia.[6][7]In countries without rubella immunization, the incidence is estimated to range between 60-220 per 100,000 cases.[8]

Screening

There is no screening test for congenital rubella syndrome.[9]

Natural History, Complications and Prognosis

History and Symptoms

Physical Examination

Congenital rubella syndrome affects multiple organ systems. The classic triad of congenital rubella syndrome consists of cataracts, sensorineural hearing loss and patent ductus arteriosus (PDA). However, many other findings are evident on physical examination, including low birth weight, skin lesions and hepatosplenomegaly.[1][10][5][11][12]

Laboratory Findings

Electrocardiogram

Chest X Ray

CT Scan

MRI

Echocardiography and Ultrasound

On echocardiography, the several cardiac anomalies associated with congenital rubella syndrome may be seen. These include patent ductus arteriosus, pulmonary artery stenosis, coarctation of the aorta, as well as septal defects (VSD or ASD).[11][13] Prenatal ultrasound, although not sensitive, is highly specific in detecting the various anomalies associated with congenital rubella syndrome. Findings such as IUGR, fetal hydrops, microcephaly, cardiac and ophthalmic defects may be seen on ultrasound.[1][6][14]

Other Imaging Findings

There are no other imaging findings associated with congenital rubella syndrome.

Other Diagnostic Studies

There are no other diagnostic studies associated with congenital rubella syndrome.

Medical Therapy

There is no specific medical treatment for congenital rubella syndrome.[15]

Surgery

The role of surgery in patients with congenital rubella syndrome consists of treating the several cardiac[16], ophthalmic[17][18] and hearing complications that arise as a result.[1][10][5][11][12]

Primary Prevention

MMR vaccination prior to pregnancy can prevent congenital rubella syndrome.[10]

Secondary Prevention

Infants with congenital rubella syndrome should be placed in isolation during their hospital stay. In addition, appropriate measures should be taken to ensure that all healthcare workers and caregivers of the infected child are immune to rubella.[3]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 De Santis M, Cavaliere AF, Straface G, Caruso A (2006). "Rubella infection in pregnancy". Reprod. Toxicol. 21 (4): 390–8. doi:10.1016/j.reprotox.2005.01.014. PMID 16580940.
  2. 2.0 2.1 2.2 Cooper LZ, Krugman S (1967). "Clinical manifestations of postnatal and congenital rubella". Arch. Ophthalmol. 77 (4): 434–9. PMID 4164540.
  3. 3.0 3.1 Center for Disease Control and Prevention https://www.cdc.gov/vaccines/pubs/surv-manual/chpt15-crs.html. Accessed on Jan 5, 2017
  4. Neu N, Duchon J, Zachariah P (2015). "TORCH infections". Clin Perinatol. 42 (1): 77–103, viii. doi:10.1016/j.clp.2014.11.001. PMID 25677998.
  5. 5.0 5.1 5.2 Ajij M, Nangia S, Dubey BS (2014). "Congenital rubella syndrome with blueberry muffin lesions and extensive metaphysitis". J Clin Diagn Res. 8 (12): PD03–4. doi:10.7860/JCDR/2014/10271.5293. PMC 4316306. PMID 25654000.
  6. 6.0 6.1 Bouthry E, Picone O, Hamdi G, Grangeot-Keros L, Ayoubi JM, Vauloup-Fellous C (2014). "Rubella and pregnancy: diagnosis, management and outcomes". Prenat. Diagn. 34 (13): 1246–53. doi:10.1002/pd.4467. PMID 25066688.
  7. "Three cases of congenital rubella syndrome in the postelimination era--Maryland, Alabama, and Illinois, 2012". MMWR Morb. Mortal. Wkly. Rep. 62 (12): 226–9. 2013. PMID 23535689.
  8. WHO (2012). "Surveillance Guidelines for Measles, Rubella and Congenital Rubella Syndrome in the WHO European Region". PMID 23762964.
  9. U.S. Preventive Services Task Force https://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=congenital+rubella+syndrome. Accessed on Jan 17, 2017.
  10. 10.0 10.1 10.2 Lambert N, Strebel P, Orenstein W, Icenogle J, Poland GA (2015). "Rubella". Lancet. 385 (9984): 2297–307. doi:10.1016/S0140-6736(14)60539-0. PMC 4514442. PMID 25576992.
  11. 11.0 11.1 11.2 Bullens D, Smets K, Vanhaesebrouck P (2000). "Congenital rubella syndrome after maternal reinfection". Clin Pediatr (Phila). 39 (2): 113–6. PMID 10696549.
  12. 12.0 12.1 Yazigi A, De Pecoulas AE, Vauloup-Fellous C, Grangeot-Keros L, Ayoubi JM, Picone O (2017). "Fetal and neonatal abnormalities due to congenital rubella syndrome: a review of literature". J. Matern. Fetal. Neonatal. Med. 30 (3): 274–278. doi:10.3109/14767058.2016.1169526. PMID 27002428.
  13. Oster ME, Riehle-Colarusso T, Correa A (2010). "An update on cardiovascular malformations in congenital rubella syndrome". Birth Defects Res. Part A Clin. Mol. Teratol. 88 (1): 1–8. doi:10.1002/bdra.20621. PMID 19697432.
  14. Cordier AG, Vauloup-Fellous C, Grangeot-Keros L, Pinet C, Benachi A, Ayoubi JM, Picone O (2012). "Pitfalls in the diagnosis of congenital rubella syndrome in the first trimester of pregnancy". Prenat. Diagn. 32 (5): 496–7. doi:10.1002/pd.2943. PMID 22495555.
  15. Cofre F, Delpiano L, Labraña Y, Reyes A, Sandoval A, Izquierdo G (2016). "[TORCH syndrome: Rational approach of pre and post natal diagnosis and treatment. Recommendations of the Advisory Committee on Neonatal Infections Sociedad Chilena de Infectología, 2016]". Rev Chilena Infectol (in Spanish; Castilian). 33 (2): 191–216. doi:10.4067/S0716-10182016000200010. PMID 27314998.
  16. Radiopedia.org https://radiopaedia.org/articles/patent-ductus-arteriosus. Accessed on Jan 17, 2017
  17. Russell HC, McDougall V, Dutton GN (2011). "Congenital cataract". BMJ. 342: d3075. PMID 21622506.
  18. Lloyd IC, Goss-Sampson M, Jeffrey BG, Kriss A, Russell-Eggitt I, Taylor D (1992). "Neonatal cataract: aetiology, pathogenesis and management". Eye (Lond). 6 ( Pt 2): 184–96. doi:10.1038/eye.1992.37. PMID 1624043.


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